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Vol. 60. Issue 3.
Pages 208-209 (January 2009)
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Vol. 60. Issue 3.
Pages 208-209 (January 2009)
Síndrome PFAPA
Síndrome PFAPA
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Suzete André
Corresponding author
Suzete.andre@hotmail.com

Corresponding author.
, Fernando Vales, Eduardo Cardoso, Margarida Santos
Servicio de Otorrinolaringología, Hospital de S. João, Porto, Portugal
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Abstract

PFAPA syndrome is characterized by periodic fever, pharyngitis, cervical adenitis, and aphthous stomatitis. The bouts of fever can last for days or even weeks. Between crises, patients remain asymptomatic for variable periods. It appears before the age of five and has limited duration (4–8 years). Its aetiopathogeny is unknown. Corticoids are the treatment of choice. Tonsillectomy has been proposed as a solution but remains controversial. We present the case of a 4-year-old girl with PFAPA syndrome who underwent tonsillectomy in January, 2008 and review the literature.

Keywords:
PFAPA syndrome
Periodic fever
Corticoid therapy
Tonsillectomy
Resumen

El síndrome PFAPA se caracteriza por fiebre periódica, faringitis, adenitis cervical y estomatitis aftosa. Los episodios febriles pueden durar desde días hasta semanas y están separados por períodos asintomáticos y de duración variable. Se presenta antes de los 5 años y tiene duración limitada (4–8 años). Su etiopatogenia es desconocida. El tratamiento más aceptado es la corticoterapia. La amigdalectomía ha sido propuesta como una solución, sin conclusiones definitivas. Presentamos el caso de una paciente de 4 años diagnosticada de síndrome PFAPA, sometida a amigdalectomía en enero de 2008 y hacemos una revisión de la literatura.

Palabras clave:
Síndrome PFAPA
Fiebre periódica
Corticoterapia
Amigdalectomía

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